Blood Disorder: Polycythemia Vera

Do your hands or feet ache all the time and you bruise too easily? So did your father? Did your grandmother soak her feet in Epsom salts and rub soothing peppermint creams on them? Maybe it’s just the old family joke of your “flat feet and thin skin,” or maybe it’s more. . .

Polycythemia Vera is a rare chronic disease of unknown origin. Other symptoms may include dry skin, itching, headaches, dizziness, blood clots or thrombosis of any kind, and sweating.

Research continues on this curious condition. It is easy to diagnose with a complete blood count (CBC), but is often missed because of fluctuations in blood counts and can be misdiagnosed as Lupus or as a thyroid disorder. Most patients are diagnosed at around age 60 and polycythemia vera is rarely diagnosed in people younger than 20 years of age. It is considered a chronic disease, but it can have fatal complications.

Polycythemia Vera is caused by a change in a blood-forming stem cell in the bone marrow. The result is overproduction of red blood cells, causing thickening of the blood and clotting.

The main treatment is regular monitoring of blood levels and phlebotomy when needed. That is, blood is withdrawn from the patient to bring the red blood cells within normal range. This procedure is done at various intervals, according to the patient’s needs. The immediate result is relief in symptoms such as headaches and profuse sweating. There are other treatment options as well.

In some patients, the disease goes into what is called the “spent” phase, in which overproduction of red blood cells are no longer an issue. The opposite problem occurs: Overproduction of white blood cells and platelets causes thinning of the blood and unexplainable bruising and pain, as well as difficulty in getting bleeding to stop.

The main treatment for the spent phase is regular blood monitoring and blood transfusions. There are a few other treatments that may help.

Careful monitoring by a hematologist (a doctor who specializes in blood disorders) and therapy often provide a normal or near-normal life quality. People with polycythemia vera are at a slightly higher risk of leukemia than the regular population, and some therapies might increase this risk.

There is some evidence to suggest that polycythemia vera may sometimes be genetic and run in families. It is important to share diagnosis with family members, so they may have their blood tested periodically and to let younger generations know of this possibility.

To learn more about polycythemia vera or other blood disorders, and the new therapies currently being studied, contact the Association of Online Cancer Resources: MPD- Net, the Leukemia & Lymphoma Society, your physician, or a hematologist.

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